Aims. The present study aimed to investigate the long-term functional results of scapulothoracic fusion using multifilament cables in patients with facioscapulohumeral dystrophy (FSHD) to identify if the early improvement from this intervention is maintained. Patients and Methods. We retrospectively investigated the long-term outcomes of 13 patients with FSHD (18 shoulders) in whom scapulothoracic fusion using multifilament cables was performed between 2004 and 2007. These patients have previously been reported at a mean of 35.5 months (24 to 87). There were eight men and five women with a mean age of 26 years. Their mean length of follow-up of our current study was 128 months (94 to 185). To evaluate long-term functional results, the range of shoulder flexion and abduction, Quick Disabilities of the Arm, Shoulder and Hand (QuickDASH) scores were analyzed with a comparison of preoperatively, interim and at the final outcomes. The fusion was examined radiographically in all. Results. The complication rate was 33% (six of 18 scapulothoracic fusions) in 13 patients, which comprised failure of fusion in four shoulders (four patients) all occurring within the first year postoperatively. In two shoulders (one patient) wound problems arose due to attribution from the cables which required shortening but the fusion developed satisfactorily. At the final examination, the mean QuickDASH score and range of movement significantly improved in all but one patient (p < 0.001, p < 0.001 and p < 0.001). In the comparison of 13 patients’ mid- and long-term results, the mean QuickDASH score decreased from 9.8 (. sd. 6.7; 3 to 26) in the third year to 9.1 (. sd. 5.6; 3 to 22) in the tenth year (p = 0.7); the mean range of shoulder flexion and abduction decreased from 129° (. sd. 22°; 90° to 160°) and 124° (. sd. 12; 100° to 150°) at the mid-term to 103° (. sd. 12°; 80° to 120°) and 101° (. sd. 8°; 80° to 120°) at the long-term, respectively (p = 0.78 and p = 0.65). Conclusion. Scapulothoracic fusion using a multiple cabling method can confer a considerable improvement in clinical and functional outcomes for most patients with FSHD after a long follow-up period. The technique requires careful execution to avoid complications. Cite this article: Bone Joint J 2018;100-B:953–6

Eleven thoracoscapular fusion operations have been done on six patients. The indication is symptomatic winging of the scapula caused by thoracoscapular muscle paresis with intact function in the deltoid. This situation almost exclusively occurs in facioscapulohumeral dystrophy. The operation is successful in achieving stability of the scapula and in greatly improving function and cosmesis. Although the course of this type of muscular dystrophy is variable, the benefits of operation have not deteriorated with progression of the disease over a maximum follow-up period of twenty-three years

Introduction: Facioscapulohumeral muscular dystrophy (FSHD) is the third most common hereditary disease of the muscle after Duchenne and myotonic dystrophy. FSHD consists in an atrophic myopathy with predominant involvement of the face, upper arms, and shoulder muscles. FSHD compromises the muscles of the scapu-lothoracic joint, and usually spares muscles of the scapu-lohumeral joint. Consequently, when the patient tries to abduct or foreword flex the shoulder, the contraction of the relatively preserved scapulo-humeral muscles produces an abnormal rotation of the scapula, that is not hold to the chest wall because of the insufficiency of the scapulo-thoracic muscles, resulting in a winged scapula and in a limited arm motion. The aim of this study is to describe an original scapulopexy and report the long term results obtained in 9 patients affected by FSHD. Material and methods: Nine patients (4 male and 5 female) affected by winged scapula in FSHD were observed and selected. Average age at surgery was 25 years (range 13–39). Surgery consisted in scapulopexy of both sides in the same surgical time. Through a skin incision on its medial border the scapula was exposed and positioned over the chest. The 5th to the 8th ribs were exposed in the part below the scapula. The position of the scapula over the chest was fixed by 4 doubled metal wires to the 5th, 6th, 7th and 8th rib. Each wire was passed anteriorly to the rib, and into a hole performed 1.5 cm from the medial border of the scapula. By tightening the wires, the scapula was fixed firmly over the chest. A figure of eight dressing that holds the shoulders back was applied immediately after surgery and maintained for six weeks. Results: The average surgical time was 65 minute for each single procedure. One patient experienced an unilateral pneumothorax the day after operation, which resolved spontaneously in 48 hours. Average follow up was 11 years (range 3–15). All patients experienced a complete resolution of the winged scapula and an improvement of range of motion of the shoulder compared to the pre operative conditions. Average pre-operative abduction was 68 and post-operative was 85. Average pre-operative flexion was 78 and post-operative 112. Discussion: This technique is easy and quick to perform, does not require grafts, reduces post surgical complications such as pneumothorax or haemothorax, and ensures good results even at considerable follow-up

Scapulothoracic fusion (STF) for painful winging of the scapula in neuromuscular disorders can provide effective pain relief and functional improvement, but there is little information comparing outcomes between patients with dystrophic and non-dystrophic conditions. We performed a retrospective review of 42 STFs in 34 patients with dystrophic and non-dystrophic conditions using a multifilament trans-scapular, subcostal cable technique supported by a dorsal one-third semi-tubular plate. There were 16 males and 18 females with a mean age of 30 years (15 to 75) and a mean follow-up of 5.0 years (2.0 to 10.6). The mean Oxford shoulder score improved from 20 (4 to 39) to 31 (4 to 48). Patients with non-dystrophic conditions had lower overall functional scores but achieved greater improvements following STF. The mean active forward elevation increased from 59° (20° to 90°) to 97° (30° to 150°), and abduction from 51° (10° to 90°) to 83° (30° to 130°) with a greater range of movement achieved in the dystrophic group. Revision fusion for nonunion was undertaken in five patients at a mean time of 17 months (7 to 31) and two required revision for fracture. There were three pneumothoraces, two rib fractures, three pleural effusions and six nonunions. The main risk factors for nonunion were smoking, age and previous shoulder girdle surgery.

STF is a salvage procedure that can provide good patient satisfaction in 82% of patients with both dystrophic and non-dystrophic pathologies, but there was a relatively high failure rate (26%) when poor outcomes were analysed. Overall function was better in patients with dystrophic conditions which correlated with better range of movement; however, patients with non-dystrophic conditions achieved greater functional improvement.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.